GUILLAIN BARRE SYNDROME “GBS” Flashcards
GUILLAIN BARRE SYNDROME
“GBS”
is the most common cause of rapidly evolving motor paresis and paralysis and sensory deficits
Individuals affected typically
reach maximal weakness within 2 to 3 weeks and spend weeks to months recovering
The most common form of GBS is
also known as acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
GBS was once classified as a single entity characterized by PNS demyelination but now it is defined by several heterogenous forms
Occurs at all ages but
peaks in frequency can be seen in young adults and in the fifth through eighth decades
Annual incidence of GBS varies from 1 to 2 cases per 100,000
the primary target of attack is
Myelin of the Schwann cell
Pathogenesis
Lesions occur throughout the PNS from the spinal nerve roots to the distal termination of both motor and sensory fibers
Myelin of the Schwann cell is the primary target of attack
An inflammatory response is initiated by circulating antibodies that bind to the surface of the myelin; lymphocytes (T cells) and macrophages are the inflammatory cells present
Demyelination occurs because macrophages responding to inflammatory signals, strip myelin from the nerves
After the initial demyelination, the body initiates a repair process
In addition to demyelination, axonal degeneration to so
Clinical Manifestations
The “classic” presentation is an acute form in which the time from onset to peak impairment is 4 weeks or less
symptoms are characterized by
a rapidly ascending symmetric motor weakness and distal sensory impairments
The first neurologic symptom is often
paresthesia in the toes
This is followed within hours or days by weakness distally in the legs
Weakness spreads to the arms, trunk and facial muscles
Flaccid paralysis is accompanied by absence of DTRs
Occasionally sensory and motor symptoms start in the arms and hands first
In 50% of cases, palatal and facial muscles become involved; even muscles of mastication may be involved
progression of symptoms
In 50% of cases, progression of symptoms generally cease in 2 weeks and 90% of cases progression ends by 4 weeks
After the progression stops, a static phase begins, lasting 2 to 4 weeks before recovery occurs in a proximal to distal progression. This recovery may take months or even years
Diagnosis
Careful clinical and neurophysiologic examinations and laboratory tests are needed
Treatment
Because GBS is believed to be an auto-immune disease, treatment has been aimed at controlling the response
Plasmapheresis – plasma is removed from circulation and filtered to remove or dilute circulating antibodies
High dose intravenous (IV) administration of immunoglobulin which is a protein the immune system normally uses to attack foreign organisms
Although most persons recover, up to
20 % can have remaining neurologic deficits. After 1 year, 67% of clients have complete recovery, but 20 % remain with significant disability.42 Even after 2 years, 8% have not recovered.
Exercise
When the person’s condition stabilizes, a therapeutic pool can be used to initiate movement in a controlled environment
A major precaution during the early treatment phase
is to provide gentle stretching and active, active-assistive exercise at a level consistent with the person’s muscle strength